CF Flashcards Preview

Pulmonary > CF > Flashcards

Flashcards in CF Deck (7):

What is the mutation that causes of CF?
What is the cause of this mutation?

deletion of delta508 in CFTR gene on chrom 7q

defective CFTR Cl- channel


How does a baby with CF present?

-salty skin
-meconium illius (no abnormalities with GI tract, poop just gets stuck due to super viscous pancreatic secretions)
-failure to thrive


What causes all the problems in CF?

thick mucous secretions createes plugs in lungs, pancreas, and liver


What problems do CF pts have?

chronic bronhcitis --> bronchiectasis
pancreatic insufficiency --> FATTY REPLACEMENT
billiary cirrhosis
fat soluble vitamin def
infertility in men


How is CF diagnosed?

high Cl- conc in sweat test


What pneumonia is assc with CF?

pneudomonas and s.aureus


What is the treatment for CF?

N-Acetylcysteine loosens plugs by cleaving disulfide bonds in mucous glycoproteins